Lupus is a chronic and prototypical systemic autoimmune disease characterized by multisystem microvascular inflammation leading to organ damage as a result of loss of tolerance to self-antigens [1-4]. Symptoms of lupus can occur in any part of the body, including the skin, heart, lungs, kidneys, joints and/or nervous system. Lupus may also be caused by a hypersensitive reaction to a medication.
The prevalence of lupus varies among geography regions and races from approximately 40 per 100,000 persons in Northern Europeans to more than 200 per 100,000 persons among blacks. In the United States, the number of patients with lupus is over 250,000 [2]. It is more frequent in African, Japanese and Chinese. The exact cause of lupus remains unknown, various factors including genetic, racial, hormonal, and environmental factors have been associated with the development of the disease [2, 5]. It is estimated that women are eight times more likely to develop either discoid or systemic lupus than men are. While the disease can people of all ages, including newborns and even the fetus, it usually develops in individuals who are 20 to 45 years old. Infections, renal failure, and cardiovascular diseases account for the majority of deaths as a result of lupus.
Although lupus is an autoimmune disease, its heterogeneous nature makes the exact mechanisms for its development unclear. There is currently no cure for lupus. Current treatments only focus on alleviating the symptoms, rather than the cause. These treatments include 1) anti-inflammatory medications (such as ibuprofen), 2) high doses of corticosteroids, and 3) immunosuppressants, such as methotrixate. While toxicities can become significant because of long-term maintenance therapy required, the disease eventually progresses to end-stage multiple organ damages and death. Although recent extensive studies have enriched our knowledge in understanding molecular pathogenesis of lupus, development of therapies for lupus has largely lagged. Despite its moderate efficacy, belimumab (Benlysta®) is the only new drug approved recently by FDA for treatment of lupus in over 50 years. Belimumab is an injectable monoclonal antibody designed to target B-lymphocyte stimulator BLyS protein, and to relieve flare-ups and pain caused by lupus, which may reduce the number of abnormal B cells thought to be a problem in lupus. Therefore, a new effective treatment of lupus is highly desired.
A need exists for a treatment for lupus that is effective and preferably treating the cause and not just the symptoms of the disease.